Most patients with appendicitis had elevated white blood cell count. It is rarely seen with leukopenic presentation in acute appendicitis. Physicians in the emergency department easily miss these cases that had normal white blood cell count and leukopenic presentation. We presented a case of a 38-year-old man who had right lower quadrant abdominal pain with fever. White blood cell count was only 1800/μL. Computed tomography showed thickening with fluid contained in the lumen of distended appendix, and emergent appendectomy was done. Accurate diagnosis of acute appendicitis is still based on the clinical history and physical examination adjuvant with imaging studies.